8.3.R2 Recommendation

Physical therapy

Published July 8, 2022

Strength of recommendation:
Conditional for intervention
Level of evidence:
Very low
How the strength and level of evidence are graded

What the strength of a recommendation means

The strength of a recommendation reflects the extent to which a guideline panel is confident that desirable effects of an intervention outweigh undesirable effects, or vice versa, across the range of patients for whom the recommendation is intended.

Strong for (↑↑) or against (↓↓) intervention
For patients: Most individuals in this situation would want the recommended course of action and only a small proportion would not.
For clinicians: Most individuals should receive the recommended course of action.

Conditional for (↑) or against (↓) intervention
For patients: The majority of individuals in this situation would want the suggested course of action, but many would not.
For clinicians: Recognize that different choices will be appropriate for different patients, and that you must help each patient arrive at a management decision consistent with her or his values and preferences.

Neither intervention nor comparison (—)
The panel did not recommend for or against the intervention.

Source: GRADE Handbook, section 6.1, Table 6.1 (GRADE uses “weak” and “conditional” interchangeably).

For the rating of the strength of the recommendation, in addition to evidence from studies in FRDA, evidence from like conditions, clinical experience and expert consensus are taken into account when published evidence is not available.

The level of evidence is based on published evidence from studies in FRDA. If there is no published evidence in FRDA, evidence from other like conditions or clinical expertise may have been used to make the recommendation – this is graded as ‘very low’ or in some cases ‘low’ level evidence. See the table below for an explanation of the symbols used to grade recommendations.

Strength of recommendation Symbol Level of evidence Symbol
Strong for intervention ↑↑ High ⨁⨁⨁⨁
Conditional for intervention ↑ Moderate ⨁⨁⨁◯
Neither intervention nor comparison — Low ⨁⨁◯◯
Conditional against intervention ↓ Very low ⨁◯◯◯
Strong against intervention ↓↓
Chapter PDF

We conditionally recommend the use of physical therapy in the first instance, over no physical therapy or medication to manage pain in individuals with Friedreich ataxia. If physical therapy is not effective, as per the previous recommendation we conditionally recommend the use of oral medication over no medication.

Question

Should physical therapy versus no intervention or versus medications be used for musculoskeletal pain with Friedreich ataxia?

Justification

Despite there being no published evidence directly related to the use of physical therapy for pain management in Friedreich ataxia, the expert authors agree that the negative effects of pain on quality of life, mobility and participation in daily activities support proactive management of pain by physical therapy. Clinical experience in both individuals with and without Friedreich ataxia suggests that physical therapy is a low-risk intervention that may be effective and might limit the reliance on medication.

Subgroup consideration

This recommendation is for individuals with Friedreich ataxia who report pain. Some individuals may have significant limitations to the type and extent of physical therapy they can do due to their neurological and possibly cardiac disease manifestations.

Evidence to recommendation table (PDF)

Part of 8.3 Pain not related to neuropathy

References (14)

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4. Ben Smail D, Jacq C, Denys P, Bussel B. Intrathecal baclofen in the treatment of painful, disabling spasms in Friedreich's ataxia. Mov Disord. 2005;20(6):758-9.

5. Berntsson SG, Gauffin H, Melberg A, Holtz A, Landtblom AM. Inherited ataxia and intrathecal baclofen for the treatment of spasticity and painful spasms. Stereotact Funct Neurosurg. 2019;97(1):18-23.

6. Kalyvas AV, Drosos E, Korfias S, Gatzonis S, Themistocleous M, Sakas DE. Intrathecal baclofen therapy for painful muscle spasms in a patient with Friedreich's ataxia. Stereotact Funct Neurosurg. 2018;96(2):127-30.

7. Koeppen AH, Mazurkiewicz JE. Friedreich ataxia: neuropathology revised. J Neuropathol Exp Neurol. 2013;72(2):78-90.

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13. Epstein E, Farmer JM, Tsou A, Perlman S, Subramony SH, Gomez CM, et al. Health related quality of life measures in Friedreich Ataxia. J Neurol Sci. 2008;272(1-2):123-8.

14. Xiong E, Lynch AE, Corben LA, Delatycki MB, Subramony SH, Bushara K, et al. Health related quality of life in Friedreich Ataxia in a large heterogeneous cohort. J Neurol Sci. 2020;410:116642.

Disclaimer, intended use and funding

Disclaimer
The Clinical Management Guidelines for Friedreich ataxia (‘Guidelines’) are protected by copyright owned by the authors who contributed to their development or said authors’ assignees.

These Guidelines are systematically developed evidence statements incorporating data from a comprehensive literature review of the most recent studies available (up to the Guidelines submission date) and reviewed according to the Grading of Recommendations, Assessment Development and Evaluations (GRADE) framework © The Grade Working Group.

Guidelines users must seek out the most recent information that might supersede the diagnostic and treatment recommendations contained within these Guidelines and consider local variations in clinical settings, funding and resources that may impact on the implementation of the recommendations set out in these Guidelines.

The authors of these Guidelines disclaim all liability for the accuracy or completeness of the Guidelines, and disclaim all warranties, express or implied to their incorrect use.

Intended Use
These Guidelines are made available as general information only and do not constitute medical advice. These Guidelines are intended to assist qualified healthcare professionals make informed treatment decisions about the care of individuals with Friedreich ataxia. They are not intended as a sole source of guidance in managing issues related to Friedreich ataxia. Rather, they are designed to assist clinicians by providing an evidence-based framework for decision-making.

These Guidelines are not intended to replace clinical judgment and other approaches to diagnosing and managing problems associated with Friedreich ataxia which may be appropriate in specific circumstances. Ultimately, healthcare professionals must make their own treatment decisions on a case-by-case basis, after consultation with their patients, using their clinical judgment, knowledge and expertise.
Guidelines users must not edit or modify the Guidelines in any way – including removing any branding, acknowledgement, authorship or copyright notice.

Funding
The authors of this document gratefully acknowledge the support of the Friedreich Ataxia Research Alliance (FARA). The views and opinions expressed in the Guidelines are solely those of the authors and do not necessarily reflect the official policy or position of FARA.